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Background: Despite the widespread use of medical cannabis, little is known regarding the safety, efficacy, and dosing of cannabis products in children with cancer. The objective of this study was to systematically appraise the existing published literature for the use of cannabis products in children with cancer. Methods: This systematic review, registered with the International Prospective Register of Systematic Reviews (CRD42020187433), searched four databases: MEDLINE, Embase, PsycINFO, and the Cochrane Library. Abstracts and full texts were screened in duplicate. Data on types of cannabis products, doses, formulations, frequencies, routes of administration, indications, and clinical and demographic details as well as reported efficacy outcomes were extracted. Data on cannabinoid‐related adverse events were also summarized. Results: Out of 34,611 identified citations, 19 unique studies with a total of 1927 participants with cancer were included: eight retrospective chart reviews, seven randomized controlled trials, two open‐label studies, and two case reports. The included studies reported the use of various cannabis products for the management of symptoms. Cannabinoids were commonly used for the management of chemotherapy‐induced nausea and vomiting (11 of 19 [58%]). In controlled studies, somnolence, dizziness, dry mouth, and withdrawal due to adverse events were more commonly associated with the use of cannabinoids. Across all included studies, no serious cannabis‐related adverse events were reported. Conclusions: Although there is evidence to support the use of cannabis for symptom management, in children with cancer, there is a lack of rigorous evidence to inform the dosing, safety, and efficacy of cannabinoids. Because of the increasing interest in using cannabis, there is an urgent need for more research on medical cannabis in children with cancer. In the last 10 years, there has been an increase in the use of cannabis‐based products (both natural and synthetic cannabinoids) to help children with cancer‐related symptoms. This systematic review summarizes 19 studies related to the use of cannabinoids for the management of cancer‐related symptoms in children. The most common indication for the use of cannabinoids in the included studies was chemotherapy‐induced nausea and vomiting. A variety of cannabinoids were used: nabilone, Δ‐9‐tetrahydrocannabinol, dronabinol, and cannabidiol. The most common route of administration was oral with capsules as a formulation. [ABSTRACT FROM AUTHOR]